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Acquired ichthyosis with hoffman’s syndrome
Correspondence Address:
B D Sathyanarayana
Dept. of Skin & S.T.D, K.R. Hospital, Mysoer, Karnataka-5700 21
India
| How to cite this article: Sathyanarayana B D, Leelavathi B. Acquired ichthyosis with hoffman’s syndrome. Indian J Dermatol Venereol Leprol 2003;69:15-16 |
Abstract
A middle aged man presented with features of acquired ichthyosis with Hoffman’s syndrome. Laboratory tests support hypothyodism. Myoedema and hypertrophy of muscles were present. Patient was previously treated for Pellagra. |
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Introduction
Hypothyrotaism may result from several causes of which autoimmune and iatrogenic are the most common. Muscular hypertrophic changes seen in hypothyroid patient is called Hoffman′s Syndrome.[1]
Malignancy like Hodgkins; drugs like cholesterol lowering and antipsychotic; autoimmune and nutritional deficiency disorders; infectious diseases like H.I.V, and endocrine disorders like hypothyroidism are the common disorders that can cause acquired ichthyosis.[2]
Case Report
A 50-year-old mule farmer, presented with complaints of generalised dryness of skin with mild itching, he had taken treatment for pellagra few years back.
The scales were present prominently over the extensor and exposed parts sparing the flexures. Palms and soles were thick and hyperkeratotic with spares body hair. Tongue was thick and enlarged. His speech was slow and slurred. He had sallow complexion.
Patient′s movements were sluggish. His calf muscles were bulky and tender. Deep tendon reflexes were normal but the ankle reflex had prolonged relaxation phase (hung-up sign). On percussion ridging of the muscles was present (myoedema).
Patient experienced a decline in memory power with gradual weakness and stiffness of limbs since few years.
Positive laboratory findings included - ESR : 80 mm/hr, serumtriglyceride : 377 mg/d1, increased TSH and decrease in T3 and T4 levels, C.P.K : 2966/liter, Electromyogram showed decreased conduction. Diagnosis of acquired ichthyosis with Hoffman′s syndrome was made.
Discussion
Muscle cramps, stiffness and weakness frequently seen in hypothyroidism are due to a primary myopathy.[3] Muscle hypertrophy is a rare occurrence in hypothyroidism. It is more commonly seen in males than females and is referred as Hoffman′s syndrome in adult and Kocher-Debre-Semelaigne syndrome in children.[4] Mounding phenomenon (myoedema) which may occur when muscle is directly percussed and slow relaxation of reflexes are associated features in Hoffman′s syndrome. Thyroid replacement may cause improvement in hypothyroid myopathy but such improvement may be incomplete in severe case.[5]
In the absence of typical exposure dermatitis in pellagra, one should always rule out the possibility of hypothyroidism.
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