A Collection of Conditions Affecting the Digits

Primary Irritant Cosmetic Dermatitis

Sunita Puri, MD
Madison, Alabama

This rash on both palms concerned a 35-year-old hairdresser who said she always wears vinyl gloves while working. She recalled using a new hair-coloring product on a client a few days earlier. Within 24 hours of applying the substance, the rash began to erupt; it worsened over the course of 2 days.

Hair dyes are among the agents that most commonly cause primary irritant cosmetic dermatitis. Other culprits are lipstick, nail lacquer, antiperspirant, and sunscreen. The severity of the rash, which varies from person to person, depends on the condition of the skin at the time of exposure, the strength of the irritant, and the duration and location of exposure. Local factors such as occlusion and perspiration that facilitate the irritant’s absorption can affect the condition’s severity.

Hairdressers are continually exposed to sensitizers—eg, hair dyes, ammonium persulfate, amphoteric surfactants, and glyceryl monothioglycolate. Reactions to these agents can occur despite the use of gloves.

Topical corticosteroids, oral antihistamines, and oral prednisone resolved this patient’s rash in 10 days. Mild outbreaks may respond to antihistamines alone.

Acrocyanosis

Yelva L. Lynfield, MD
Cedarhurst, New York

Cyanosis involving the hands and toes of a 12-year-old girl was first noted 3 months before medical evaluation was sought. Her fingers and palms were red, the nail beds were cyanotic, and her palms were cold and sweaty. The girl’s toes were cyanotic when she was standing. Her hands and feet reverted to a normal color when the limbs were elevated. The rest of the physical examination findings were normal.

This presentation is typical of acrocyanosis, a benign condition most common in teenaged girls. Localization to the extremities and no involvement of the lips can help differentiate acrocyanosis from more serious forms of cyanosis. The persistent rather than intermittent nature of this condition and the relatively mild symptoms help rule out Raynaud disease.

Since acrocyanosis intensifies with exposure to cold, this patient was advised to wear gloves and warm socks in cold weather.

Idiopathic Raynaud Disease

Robert P. Blereau, MD
Morgan City, Louisiana

In January, a 14-year-old girl presented with purple coloration of the distal toes. During the week and a half before presentation, she had redness and swelling just proximal to the toenails. She had no history of injury to the toes. The digits were cool to the touch and were painful when pressure was applied. Shortly before the onset of toe discoloration, she had experienced anxiety and depression after her mother and father’s divorce.

Physical examination findings were otherwise normal. The patient had no signs or symptoms of disease, particularly of connective tissue disease, and no ulcerations.

Results of laboratory studies, including a complete blood cell count, antinuclear antibody test, rheumatoid factor test, anticyclic citrullinated peptide antibody test, and uric acid test, as well as a chest radiograph, were all normal. The patient was referred to a pediatric rheumatologist who agreed with the diagnosis of idiopathic Raynaud disease.

Raynaud phenomenon, an exaggerated physiologic response to cold with no apparent cause (idiopathic or primary Raynaud disease) often begins in adolescence and primarily manifests as asymmetrical disease without ulceration or gangrene. In contrast, secondary Raynaud disease usually develops at an older age and is associated with an underlying condition such as a connective tissue disease, eating disorder, obstructive arterial disorder, or drug use; it also often manifests with ulcerations and gangrene. Secondary Raynaud disease occurs in 80% to 90% of persons with systemic sclerosis (it is the presenting symptom in 30%). Patients with migraine and variant angina frequently have Raynaud disease.

Distinguishing between the 2 types of Raynaud disease can be challenging. Microscopy of the nail bed can help identify distorted, widened, and irregularly looped capillaries, which are characteristic of secondary Raynaud disease. In primary Raynaud disease, laboratory test results often are normal, and patients lack a family history of the condition.¹

A hyperactive sympathetic system is thought to cause severe vasoconstriction of the distal arteries of the digits and, rarely, the earlobes, tip of the nose, and penis. The decreased blood flow can cause sensations of coldness, pain, and paresthesia. The skin changes vary from pallor to cyanosis to erythema.

The female to male ratio is 5 to 1.² Fingers are affected more than toes, which are affected in 40% of cases.² Primary Raynaud disease is more common than secondary. It is milder and causes fewer complications; loss of part of a digit occurs in less than 1% of patients.²

Immediate treatment for acute Raynaud disease is physical warming of the body parts. Topical application of nitroglycerin ointment may be useful in severe cases. This patient was offered nifedipine 10 mg 3 times daily for pain or burning. Other medications, such as α-blockers, angiotensin-receptor blockers, antiplatelet drugs, topical vasodilators, intravenous prostaglandins, and onabotulinumtoxin A injection, can be used depending on the cause.³ Preventive measures include avoidance of triggers (cold, vibration, and stress) and discontinuation of smoking, caffeine, stimulants, and vasoconstrictor ingestion.

This patient’s symptoms resolved in the spring when the weather became warmer.

References:

1. Miller LM. Scleroderma and Raynaud phenomenon. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, PA: Saunders Elsevier; 2007:1024-1027.
2. Varga J. Systemic sclerosis (scleroderma) and related disorders. In: Fauci AS, Kasper DL, Longo DL, et al, eds. Harrison’s Principles of Internal Medicine. 17th ed. New York, NY: McGraw Hill; 2008:2096-2106.
3. Hinshaw M, Lawrence-Hylland S. Connective tissue disorders. In: Bope ET, Kellerman RD, eds. Conn’s Current Therapy 2016. Philadelphia, PA: Elsevier; 2016:619-623.

Subungual Hematoma and Onychomycosis

Steven Q. Wang, MD
Chatham, New Jersey

A 67-year-old woman presented for a routine skin examination. The patient had no personal or family history of melanoma, but she had had surgeries to remove 2 squamous cell cancers on the cheek and forehead. A quick clinical examination revealed a brown and yellow pigmentation in the left great toenail (Figure 1). 

The patient did not remember the duration of the lesion, and she denied any history of recent trauma.

Upon close inspection with dermoscopy (Figure 2), the pigmentation was noted to only involve the distal and middle nail, but not the proximal portion of the nail. There were red, maroon, and dark blue colors in the proximal portion of the pigmentation. Furthermore, there were reddish dots at the peripheral edge of the pigmentation and red lines in the distal portion of the pigmentation. Collectively, these dermoscopic findings were suggestive of subungual hematoma. In the distal portion of the pigmentation, the colors were predominantly yellow and gray, suggesting the diagnosis of onychomycosis.

Biopsy and fungal culture were not performed. Three months later, the subungual hematoma resolved spontaneously (Figure 3), but signs of onychomycosis still were present. The patient declined treatment with oral terbinafine.

Brachymetacarpia and Brachymetatarsia

Daniel Ivan, MD
University of Texas Medical School at Houston

A 15-year-old girl presented with her mother for a sports preparticipation physical examination. She was on the high school soccer team and also had been on the cross-country team for the past several years. She had no physical complaints.

Physical examination results were within normal limits except for a bilateral shortening deformity of the fourth toes, which slightly overlapped the adjacent fifth toes (Figure 1). According to her mother, the girl was noted to have had changes in her toes’ appearance since early childhood. Nevertheless, the changes were not interfering with her usual (and often strenuous) daily activities. A careful examination of her hands also revealed bilateral shortening of the fifth fingers, with questionable shortening of the thumbs (Figure 2). The girl’s maternal aunt had a similar finding in her toes but not in her fingers.

The patient’s past medical history was unremarkable. She was the product of a full-term uncomplicated pregnancy. She had never been hospitalized or had suffered any significant injuries. She started menstruating at age 12 years, and her menses were regular. She was of average height and weight. Her academic performance was that of an honor-roll student.

A diagnosis of brachydactyly was considered, and radiographs were obtained. The hand radiographs showed bilateral shortened fifth metacarpal bones and bilateral shortened fifth fingers middle phalanges (Figure 3). The foot films showed bilateral shortened fourth metatarsal bones (Figure 4). The final diagnosis was bilateral brachymetacarpia and brachymetatarsia.

Brachydactyly is the term for shortening of the fingers or toes (from the ancient Greek brachys, short, and daktylos, finger) resulting from underdeveloped, absent, or abnormally shaped phalanges, metacarpals, or metatarsals.¹ It may involve single or multiple bones in individual digits, or single or multiple bones in multiple digits.¹ Brachymetacarpia is the specific term when the metacarpal bones are involved, while brachymetatarsia refers to the shortening of metatarsals.

Brachydactyly can present either as an isolated malformation or as part of a chromosomal abnormality or a complex malformation syndrome, such as Turner syndrome, pseudohypoparathyroidism, or Cohen syndrome.¹

The diagnosis is clinical and radiologic. Bell’s classification of brachydactyly² delineates 5 major types (A through E) and 3 subtypes of type A (A1, A2, and A3).

Isolated brachydactyly usually is transmitted in an autosomal dominant pattern with variable penetrance.³ The literature is scarce on the incidence of concomitant brachymetacarpia and brachymetatarsia.⁴ Mild brachydactyly often is insignificant in terms of functional prognosis. More severe degrees may cause cosmetic concerns or even functional impairment. Referral to an orthopedic surgeon or podiatrist may be indicated.

In our patient’s case, no further workup was considered. Her brachydactyly was categorized as type E in Bell’s classification system. The physical findings were isolated, and no other historical or clinical data suggested a genetic or syndromic disorder. She was referred to a podiatrist for further evaluation. 

References:

1. Everman DB. The brachydactylies. In: Stevenson RE, Hall JG. Human Malformations and Related Anomalies. 2nd ed. New York, NY: Oxford University Press; 2006:968-983.
2. Bell J. On brachydactyly and symphalangism. In: Penrose LS, ed. On Hereditary Digital Anomalies. London, England: Cambridge University Press; 1951:1-31.
3. Temtamy SA, Aglan MS. Brachydactyly. Orphanet J Rare Dis. 2008;3:15. doi:10.1186/1750-1172-3-15.
4. Kirkos JM. Idiopathic symmetrical shortening of the fourth and fifth metacarpal and metatarsal bilaterally: a case report. Acta Orthop Belg. 1999;65(4):532-535.